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Complex Regional Pain Syndrome (formerly known as Reflex Sympathetic Dystrophy and/or Causalgia)

December, 2000

Complex Regional Pain Syndrome is one of the most difficult and frustrating problems in the field of personal/injury/worker's compensation.  Individuals with this disorder are often extremely challenging from the case management perspective. The following article gives a "professional patient's" perspective on this problem (excerpted from Clin J Pain, 12(2):90-93, 1996):

The author, a psychologist in a pain control center, was asked by the editor of The Clinical Journal of Pain to relate her personal experience with neuropathic pain. Her chronic pain began six years previously when her car was rear-ended by a large tractor-trailer truck. After several weeks of traction, rest, and a cervical collar, healing began. One year later she was re-injured in a fall and in addition to the cervical injury also injured her lumbar spine. She experienced right upper and lower extremity numbness, loss of fine motor skills in the right hand, and difficulty walking. Despite her neurosurgeon's urging to maintain strict bed rest for two months, she put herself on a walking program to prevent muscle atrophy. She continued to have chronic pain with occasional flare-ups.

Several months later, in the process of building a patio lounge chair, her already weakened spine was again injured. She required surgery, including a fusion at C4-5, along with removal of a ruptured disc and removal of several fragments from the spinal canal. Presurgical nerve damage caused prolonged motor weakness and numbness. Also, when the bone graft was removed from the iliac crest, injured nerves left her with neuropathic pain in the left thigh and leg. During the subsequent healing months, the author learned personally about definitions of complex regional pain syndrome (CRPS), allodynia, dysesthesia, hyperesthesia, and about listening to patients in pain and believing their story. She discusses several "do's and don'ts" for surgeons and for patients, related to her personal experience. Probably the most valuable advice for pain management professionals is to listen to your patients. Ask them what is wrong and they will tell you. It is common to ask patients questions which relate to the experience of chronic pain, but do not seem to relate to CRPS symptoms. For example, "When is your pain worse?" "What positions worsen pain?" While these activities do relate to chronic pain, there is no mention of experiences that affect neuropathic pain such as encountering a sudden blast of water while in the shower, walking into an air conditioned room, or walking outside on a windy day. Also, questionnaires contain descriptions of chronic pain symptoms but not CRPS symptoms such as crawling ants, stinging bees, and soft cotton being rubbed across one's skin. The author describes the feeling of cold air from air conditioning or wind contacting her skin feeling like lightning-sharp goose bumps like cactus spikes. She experienced an intense burning, stinging sensation as though a swarm of angry yellow jackets was stinging profusely and unrelentingly. Areas of her skin felt hot and cold simultaneously. She had a feeling of soft puffs of cotton containing shards of razor-sharp steel being rubbed agonizingly slowly over her skin where the bone graft was taken. Also, she described a feeling of a soft feather being rubbed tortuously slowly and softly over her skin. These are symptoms which the author feared no one would believe if she actually described how they felt.

As a psychologist, she never actually disbelieved, but did doubt patients who told her they hurt too badly to comply with their relaxation and visual imagery exercises. She never disbelieved, but did doubt patients who reported that their pain "moved" or increased after receiving a nerve block. As a patient, she learned what they meant.

She emphasizes the importance of treating patients with empathy, respect, and explanations of treatments. One of the most beneficial things she gained by being a patient is to listen to her patients and try to actually hear what they are telling her, instead of listening for what she thinks the patients should say in order for her to impose a known treatment on a familiar sounding problem. She believes persons stereotyped as "professional" patients may simply be patients seeking professional help.

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What is CRPS?

The concept and nomenclature of reflex sympathetic dystrophy (RSD) has changed.  The syndrome is being reclassified by the International Association for the Study of Pain (IASP) as complex regional pain syndrome (CRPS) Types I and II. Gone will be RSD and causalgia classifications and in their stead will be CRPS Type I and Type II, respectively.

The conditions usually follow injury and have distal abnormal findings which exceed the expected course of the original injury both in magnitude and duration and often result in significant motor impairment.

CRPS Type I (RSD) usually follows a noxious event, is not limited to the distribution of a single peripheral nerve, and is disproportionate to the inciting event. It is associated at some point with edema, changes in skin blood flow, abnormal pseudomotor activity in the area of the pain, and allodynia or hyperalgesia. Usually it is distal to the injury. It involves the peripheral and possibly the central nervous system (CNS). Diagnostic criteria are: 1. an initiating noxious event; 2. continuing disproportionate pain; 3. signs of local or distal skin changes; and 4. exclusion of other causes for the pain and changes.

CRPS Type II, (causalgia) usually occurs immediately after the original insult but may be delayed. Most frequent nerves involved are the median, sciatic, tibial, ulnar, and, very rarely, the radial nerve. Pain is described as constant, burning, exacerbated by light touch, stress, temperature change or movement of the limb, visual and auditory stimuli, or emotional upheavals. Allodynia and hyperalgesia may not be limited to a specific nerve distribution. Skin temperature and edema parallel RSD, but effects may spread proximally and frequently motor function is disturbed. Diagnostic criteria are: 1. presence of continuing pain, allodynia, or hyperalgesia after a nerve injury, not necessarily limited to the distribution of the injured nerve; 2. skin changes; and 3. lack of other causes for the symptoms. All three criteria must be met.

Current treatment for Type I and other neuropathic pain syndromes is largely determined by trials of therapy and frequently trials of multiple concurrent therapies. Psychological treatment is necessary because there is a problem with suicide in some of these patients. The cost of treatment, since it may take long periods, is formidable for some patients. Types I and II may be treated according to an analgesic ladder. Step one includes treatment with opioids, transcutaneous electrical nerve stimulation, topicals, tricyclic antidepressants, psychotherapy, education, physical therapy, and occupational therapy. Step two may include those in step one, but adds regional or systemic sympathetic blockade, treatment of emotional components of pain, IV regional blocks, peripheral block-infusion, carbamazepine, baclofen, clonidine, steroids, non-steroidal anti-inflammatory drugs, mexiletine, or other drug trials. Step three may include the above and sympathectomy or sympatholysis, peripheral nerve decompression or lysis, dorsal column stimulation, peripheral nerve stimulation (PNS) or intrathecal/epidural analgesia. This ladder is supported by clinical judgment rather than statistically valid outcome data. With CRPS, the sooner the diagnosis is made and treatment begun, the more successful the outcome.

In summary, CRPS is complex and difficult to treat. Therapy may be necessary for prolonged periods and psychological as well as physical approaches may be necessary.

Excerpted from:  Seminars in Anesthesia, 15(1):70-87, 1996.
 

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